2016 Volume 37 Issue 2 Pages 151-156
Aarskog syndrome is a rare X-linked recessive disease characterized by typical facial features, a shawl scrotum, and short stature. The phenotype varies among cases.
Here, we report the case of a 5-year-old boy with Aarskog syndrome and mental retardation who underwent dental treatment under general anesthesia. Oral examination revealed many decayed teeth and malocclusion （deep bite） caused by hypoplasia of the lower mandible and the small available arch length of the mandible. The patient was extremely fearful of dental treatment as he was mentally retarded and was unable to understand and cooperate with the dental treatment. Therefore, treatment was administered under general anesthesia.
Anesthetic considerations included difficulties in tracheal intubation due to lower facial deformities. Anesthesia was induced and maintained using sevoflurane and nitrous oxide in oxygen. Mask ventilation was easily performed：thereafter, we attempted laryngoscopy using a Miller blade and decided to intubate using the conventional method. Tracheal intubation was successful after the administration of a muscle relaxant. Dental treatment included composite resin filling, endodontic treatment, metal crown restoration, occlusal adjustment, and extraction of severely decayed deciduous molars. We conducted three sessions of treatment under general anesthesia. In all three times of treatment, general anesthesia was well maintained, the treatment was uneventful, and no perioperative complications occurred.
After these treatments, we started a monthly follow-up regimen to maintain his oral hygiene. For patients with mental retardation, repeated dental check-ups enable the patient to adapt to the conventional dental check-ups, and regular check-ups help in preventing dental diseases. In addition, for parents of children with mental retardation, guidance regarding oral hygiene and daily oral care is also important.
